Rudimentary Horn Pregnancy with Neonatal and Maternal Survival

John P. O'Grady, MD, and Frank A. Salem, MD Los Angeles, California

Pregnancy in a rudimentary uterine horn is an unusual event that fetal cardiac arrythmia, and noted hy- often terminates early in gestation with catastrophic hemorrhage. dramnios and a posterior placenta. Under rare circumstances an intact rudimentary horn or a secondary Normal and progressive fetal growth abdominal pregnancy from a ruptured horn continues to term. was documented by serial biparietal measurements. At the 36th week of These cases present with pain or anemia, an uncertain or abnor- gestation, the patient was seen for a mal fetal lie, and may be complicated by symptoms of preeclampsia. complaint of spotting and discharge but Physical examination, and ultrasonic and radiographic studies lead the physical examination was recorded to the correct diagnosis. Rapid surgical intervention can result in as "normal." An attempt at real-time fetal and maternal survival with preservation of fertility. ultrasonic biparietal measurement in the 38th week was unsuccessful. At 39 weeks gestation, the patient Organogenesis of the paired Mulle- Clinical History was admitted after complaining of rian system is occasionally defective A 17-year-old woman, a nullipara progressively severe abdominal pain and produces multiple structural ab- with an estimated gestational age of 21 and intermittent cramping of 24-hours normalities.1'2 One such unusual mal- weeks, was referred to our hospital for duration. formation is a rudimentary or non- ultrasonography from an outlying The patient's vital signs were nor- communicating uterine horn. Preg- clinic. Her initial physical examination mal except for a tachycardia of 110 nancy in a rudimentary horn is rare and and medical and family history were bpm. The hemoglobin level was 11.5 frequently terminates early in gestation normal. Laboratory evaluation docu- gm; serum electrolytes and coagulation with rupture and catastrophic hemor- mented AS hemoglobin; all other profile were normal. She now com- rhage. 1'3'4 The diagnosis of this condi- studies were normal. plained of constant discomfort aggra- tion is difficult and severe fetal and Fetal cardiac arrythmia, atypical vated by fetal movements. The esti- maternal morbidity common. In ex- intracranial echoes, and hydramnios mated fetal size was 2,500 gm and the ceptional cases the pregnancy may ad- were noted by ultrasound scan with a fetal heart rate (FHR) was 150-160 bpm vance into the third trimester with a vi- gray scale unit. A fetal anomaly was and regular. The position was unstable, able fetus. This report reviews the lit- suspected, and the patient was referred transverse to oblique breech, and the erature, presents the seventh case of to the High Risk Service. Fetal ECG fetus could be outlined with ease ex- rudimentary horn term gestation with obtained with sensors on the maternal cept for the fetal head which lay be- neonatal and maternal survival, and abdomen documented an irregular neath the left costal margin. Any pal- discusses diagnosis and management. rhythm of 130-160 beats per minute pation of the fetus caused marked dis- (bpm). The diagnosis of a fetal cardiac tress to the mother. There was no conduction defect or heart block was bleeding or vaginal discharge. On entertained. bimanual examination the cervix was Transient abdominal pain of mod- posterior, 1 cm dilated, and uneffaced. From the Department of Obstetrics and Gyne- erate intensity was reported between A smooth, nontender, soft pelvic mass cology and the Department of Pathology, Mar- tin Luther King, Jr. General Hospital and the 22nd and 24th weeks. This resolved of lOx 12 cm continuous with the cervix Charles R. Drew Postgraduate Medical School, Los Angeles, California. Requests for reprints spontaneously. A breech presentation was felt. Real-time ultrasonic scan was should be addressed to Dr. John P. O'Grady, persisted after the 28th week. Repeat attempted but neither the chorionic Department of Obstetrics and Gynecology, Charles R. Drew Postgraduate Medical School, ultrasonic examinations in the 28th, plate nor the amniotic sac could be vis- 12021 Wilmington Ave, Los Angeles, CA 90059. 30th, and 32nd weeks demonstrated the ualized clearly.

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978 863 4 4

A~~~~ Figure 2. Lateral radiograph dem- onstrating cranial overlap of the maternal spine (Weinberg's sign).

Figure 1. Longitudinal midline ultrasonogram revealing pelvic mass (a) and ab- sence of uterine wall between fetus and bladder (b).

I1a

Figure 3. laparatomy.IOperative findings at

Gray scale ultrasound demonstrated fluid with a segment of omentum The pathologist described a 14 close approximation offetal parts to the loosely adherent to the fetal head. The x13xlO cm, 790 gm mass, consisting maternal bladder and the apparent ab- child, a 2,810 gm normal male (APGAR of tube, ovary, and hemiuterus with sence of a uterine wall surrounding the 5/8) was delivered as a breech and sub- implanted placenta. The myometrium fetus. These scans and a retrospective sequently did well. was hypertrophied and contained many analysis of earlier ultrasound studies The placenta was implanted in a dilated vascular channels. The umbili- revealed a pelvic mass, thought to rep- cupshaped mass arising from the left cal cord was normal and the placenta resent the uterus (Figure 1). A flat plate wall of a normal appearing uterus. Ini- was unremarkable, with scattered fi- radiograph of the abdomen showed the tially, the impression was of a placental brinoid necrosis and normalvilli. Careful fetus in a "sprawled" transverse lie. implantation on the posterior uterine gross and microscopic examination On lateral films (Figure 2) there was fundus. Closer examination revealed an failed to show any evidence of an overlap of the fetal skull to the maternal apparent bicornuate uterus with the endometrial cavity at the margin of re- spine (Weinberg's sign) and close prox- gestation arising from a rupture in the section, documenting the existence of a imity of the small parts of the infant to left horn (Figure 3). noncommunicating rudimentary horn. the maternal abdominal wall. The gravid hemiuterus with attached During the postoperative period, Laparatomy was promptly performed round ligament, tube, and ovary was the patient developed transient hyper- under general anesthesia with the excised. There was no connection to tension with proteinuria and hyperre- preoperative diagnosis of abdominal the endometrial cavity of the remaining flexia. She rapidly improved and was pregnancy. right cornua and a corpus luteum was discharged home with her infant on the The fetus was lying free in the not identified. Prior to closing the inci- sixth postoperative day. Examination peritoneal cavity in several hundred sion, two normal kidneys were pal- of the infant was normal, as was a sub- milliliters of bloody amniotic/peritoneal pated. sequent electrocardiogram.

864 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978 Table 1. Cases of Rudimentary Horn Pregnancy with Neonatal and Maternal Survival Calculated Fetal Gestational Secondary Weight Gravida/ Age Anatomy Abdominal (gm) Author Age Para (weeks) Described History Pregnancy and Sex

Geipel5 23 2/1 33 Unicornuate uterus Maternal 'eclampsia' No 1,700 1950 Atretic accessory horn Abdominal pain female Laparatomy for rupture Scholtz6 25 2/0 41 Bicornuate uterus Hypertension No 2,720 1951 Noncommunicating Vaginal bleeding male rudimentary horn Normal IVP Subsequent-term gestation Bourgeois7 24 2/1 39 Unicornis unicollis Anemia, Yes 2,793 1952 Uterus sessile Abdominal pain male Accessory horn Failed induction Heinonen4 23 1/0 41 Bicornuate uterus 'Nephropathia gravidarum' No 2,150 1961 Atretic accessory horn Albuminuria female (bicornis uno Normal 'urinary tract' latere rudimentarius) Subsequent-term gestation O'Leary8 21 1/0 35 Uterus bicornis Anemia Yes 2,637 1963 Unilateral rudimentarius Second trimester abdominal pain (sex un- unicollis Recurrent third trimester specified) Pain with nausea Jarrell9 27 2/1 44 Bicornuate uterus Hypertension No 3,686 1977 Noncommunicating Hyperreflexia male rudimentary horn Postmature infant Left maternal kidney absent Primary dysmenorrhea history O'Grady 17 1/0 39 Bicornuate uterus AS hemoglobin Yes 2,810 1978 Noncommunicating Second trimester abdominal pain male rudimentary horn Recurrent third trimester pain Post delivery transient hypertension Albuminuria

Discussion In retrospect it seems unusual that minology and reference to the original an abnormality was not suspected ear- illustrations or operative and patholog- In this case, the gestation developed lier. In fact, the initial ultrasound ical descriptions is necessary, as ap- initially in a rudimentary uterine horn. studies did document a pelvic mass. parently identical anatomy may receive While speculative, it is probable that But, the initial interpretation of the differing descriptive names. the second trimester report of abdomi- films concentrated more on the diag- Three similar features characterize nal pain marked the onset of spontane- nosis of presumed fetal anomalies. A these seven patients: first, their youth ous rupture of the rudimentary horn maternal problem was not suspected and parity; second, the incidence of with intraperitoneal herniation of the until the patient's symptoms forced toxemic symptoms; and third, the suc- fetus and membranes. After this more critical analysis. cess of subsequent fertility. The episode, the patient had only minor Six similar case reports were dis- patients are all young and are of complaints until approximately 48 covered in the literature4-9 (Table 1). low parity. Five of seven patients hours prior to admission when the O'Leary's review8 mentions one addi- experienced hypertension and/or al- membranes probably ruptured, placing tional case originally described by buminuria during gestation and in one the fetus in direct contact with the ab- Serejnikoff"° which has been excluded case, toxemia was diagnosed.5 dominal viscera. Pain with fetal move- as the infant, although born alive, The reason for this frequency of ment and generalized abdominal dis- rapidly expired. Comparison of cases is preeclamptic symptoms is obscure. comfort followed. confused by the lack of standard ter- Pregnancy-induced hypertension has

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978 865 been reported with abdominal ges- nancy suggests that unusually fertilized need for exploration. Therapy is surgi- tation'112 but it has not been an impor- ova may also traverse the peritoneal cal and conservative so as to maintain tant finding in reviews of extrauterine cavity. fertility. pregnancyll1'3-18 although Beachman19 Because of its rarity and anatomic reports a single case. Perhaps the criti- variation, the diagnosis of rudimentary Acknowledgement cal factor is the site ofplacental implan- horn pregnancy is not usually made The author is grateful to Caroline Yeager, MD, Department of Radiology, Martin Luther tation. It may be that rudimentary prior to laparatomy. Less than 10 per- King, Jr. General Hospital for assistance in the horns provide a poor base for placental cent proceed into the third trimester. interpretation of ultrasonic scans. development favoring ischemia and a As most rupture early in pregnancy, progression toward toxemia. Without these initial cases are difficult to sepa- Literature Cited 1. Jarcho J: Malformations of the uterus. further data, the importance of this rate clinically from ruptured interstitial Am J Surg 71:106-166, 1946 2. Piquan G: Les uterus doubles: association remains speculative. Two gestations.1 Anatomie et developpment. Rev Gynecol patients in this series had subsequent The unusual term pregnancies may 15:401-466, 1910 3. Wahlen T: Pregnancy in non- term gestations indicating that the re- present with an abnormal lie, often ac- communicating rudimentary uterine horn. Acta maining hemiuterus can function nor- companied by pain and anemia, or as a Obstet Gynecol Scand 51:155-160, 1972 4. Heinonen J, Relander M: Pregnancies mally. Thus, at laparatomy, maximal failed induction. If extrauterine preg- in an accessory horn of the uterus. Ann Chir et conservation of normal structures is nancy exists, ultrasound and radio- Gynaec Fenniae 50:210-219, 1961 5. Geipel K: Ruptur eines atretischen desirable. graphic studies may establish the correct rudimentaren Nebenhornes im 8 bis 9 diagnosis prior to laparatomy. Schwangerschaftsomonat. Zentralbl Gynak While more than 325 cases of 72:1359-1363, 1950 rudimentary horn pregnancy have been Ultrasound may document an ab- 6. Scholtz M: A full-time pregnancy in a rudimentary horn of the uterus. J Obstet reported, the condition is unusual.20 dominal or pelvic mass separate from Gynaec Br Commonw 58:293-296, 1951 Rudimentary horn pregnancy is ten the fetus that can be identified as the 7. Bourgeois GA, Shapiro MW: Abdomi- nal pregnancy secondary to rupture of ac- times less frequent than abdominal uterus.24 It may also be possible to cessory uterus with survival of mother and in- pregnancy, approximately one in show the absence of uterine wall be- fant. N Engl J Med 247:84-87, 1952 8. O'Leary JL, O'Leary JA: Rudimentary 140,000 deliveries.8 Due to the limited tween the fetus and the bladder. Both horn pregnancy. Obstet Gynecol 22:371-375, size of rudimentary most gesta- findings were demonstrated in this case 1963 horns, 9. Jarrell J, Effer SB, Mohide PT: Preg- tions terminate with early rupture or (Figure 1). nancy in a rudimentary horn with fetal salvage. Am J Obstet Gynecol 127:676-677, 1977 death of the fetus.3 Rarely, a horn may In cases of rudimentary horn it is 10. Serejnikoff S: Ein Fall von Coeliotomie dilate to successfully accommodate a also possible to separate the gravid wegen Schwangershaft im rudimentaren Horne eines Uterus bicornis bei lebensfahiger Frucht. term gestation or, as in this case, rup- from nongravid horn by scanning, al- Mschr Geburtsh Gynak 8:232-238, 1898 ture with formation of a secondary ab- though it is rare that this opportunity is 11. Renard R, Boury-Heyler C, Leissner P, et al: Les grossesses abdominales apres le dominal pregnancy. likely to present itself. sixieme mois. Gynecol Obstet (Paris) 68:297- In secondary abdominal pregnancy, 318, 1969 Rudimentary horns occur em- 12. Anderton KJ, Duncan SL, Lunt RL: Ad- bryologically with failure of normal fu- radiographic signs are multiple. 18,25.26 vanced abdominal pregnancy with severe preeclampsia. Br J Obstet Gynaecol 83:90-93, sion of the paired Mullerian tubes. 1.2l4,21 The most useful findings are the close 1976 A variety of malformations is possible, approximation of the fetus to the ma- 13. Ware HH: Observations on thirteen cases of late extrauterine pregnancy. Am J to include communicating accessory ternal abdominal wall in a lateral radio- Obstet Gynecol 55:561-582, 1948 horns and separate atretic cornua.2 As graph and the unusual lie of the infant. 14. King G: Advanced extrauterine preg- nancy. Am J Obstet Gynecol 67:712-740, 1954 with other Mullerian anomalies, renal Overlap of the fetal skull behind the an- 15. Mitra SM: Advanced extrauterine preg- deformities are also terior surface of the maternal lumbar nancy. Calc Med J 39:43-52, 1942 frequently pres- 16. Naidu PM, Reddy UN: Advanced ex- ent.1 2 Only 10-15 percent of rudimen- vertebrae, while often mentioned as a trauterine gestation. J Obstet Gynaecol Br pathognomonic sign of abdominal ges- Commonw 67:843-847, 1960 tary horns connect to the normal 17. O'Sullivan JF: Advanced extrauterine uterine cavity; thus, the mechanisms of tation, apparently may also occur in pregnancy. lr J Med Sci 2:27-30, 1969 intrauterine gestation.26 Hysterography 18. Dixon HG, Stewart DB: Advanced ex- pregnancy and menstruation cannot trauterine pregnancy. Br Med J 1103-1111, 1960 easily be explained.4 Curiously, many and angiography have also been used to 19. Beachman WD, Hernquist WC, Beachman DW, et al: Abdominal pregnancy at of these patients may not complain of make the diagnosis but these are inva- Charity Hospital in New Orleans. Am J Obstet dysmenorrhea and endometriosis has sive techniques entailing greater risk.27 Gynecol 84:1257-1270, 1962 20. Cohn FL, Goldenburg RL: Term preg- rarely been reported as an associated nancy in an unattached rudimentary uterine While the occurrence horn. Obstet Gynecol 48:234-236, 1976 finding.22'23 of 21. Crosby WM, Hill EC: Embryology of the pregnancy attests to the functional ca- Mullerian duct system. Obstet Uiynecol Conclusion 20:507-515, 1962 pacity of the rudimentary horn, the fate 22. Lewis BV, Brant HA: Obstetric and of normal menstrual flow in these Term pregnancy in a rudimentary gynecologic complications associated with Mullerian duct abnormalities. Obstet Gynecol patients simply remains obscure. uterine horn is an unusual event. Most 28:315-322, 1966 Pregnancy occurs either through a cases rupture early in pregnancy and 23. Carpenter RJ, Jamesen WJ: Uterus bicornis unicollis with rudimentary horn. Am J connecting passage to the rudimentary are clinically indistinguishable from Obstet Gynecol 63:206-208, 1952 horn, or via transperitoneal migration.6 cornual or ectopic gestations. On oc- 24. Kobayashi M: Illustrated Manual of Ul- trasonography in Obstetrics and Gynecology. In most cases, the corpus luteum is on casion a pregnancy may proceed to Tokyo, Igaku Shoin Ltd, 1974, pp 172-177 the same side as the gestation, indi- 25. Weinberg A, Sherwin AS: A new sign in term in an intact horn, or rarely, in the roentgen diagnosis of advanced ectopic preg- cating that transperitoneal passage of peritoneal cavity as a secondary ab- nancy. Obstet Gynecol 7:99-101, 1956 26. Cockshott WP, Lawson J: Radiology of spermatozoa may be the primary dominal pregnancy following rupture. advanced abdominal pregnancy. Radiology mechanism for conception.8 The rare At term failed induction, peculiarities in 103:21-29, 1972 27. Lawson JP, Myerson DA, Myerson PJ: existence of a contralateral corpus fetal lie and the finding of anemia with Abdominal pregnancy. Br J Radiol 49:723-726, luteum with rudimentary horn preg- maternal complaints of pain suggest the 1976

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